Introduction: Biliary obstruction is usually caused by choledocholithiasis. However, in some circumstances, alternative or concurring unusual ethiologies such as portal hypertensive biliopathy (PHB) must be considered.

Clinical case: We present the case of a 36-year-old female complaining of jaundice and pruritus. Liver function tests were compatible with biliary obstruction and the ultrasound scan of the abdomen showed dilatation of the intrahepatic biliary ducts, a dilated common bile duct (CBD) and biliary calculi. The computed tomography of the abdomen revealed a portal cavernoma encasing the CBD.

Discussion: Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.

Jaundice is the most frequent presentation of liver and biliary disease and the diagnosis of obstructive jaundice can usually be established with simple laboratory tests and ultrasonography.Peep Mia Botas Toe Pretas Têxteis Curtas Shellie 1 Choledocholithiasis is by far the most common cause of biliary obstruction but other important conditions to consider are biliary or extrabiliary malignancy, pancreatitis, non-neoplastic biliary strictures and parasites.Toe Peep Pretas Shellie Têxteis Botas Mia Curtas 2 However, as evidenced by our case report, imaging investigations may bring to light an uncommon cause of biliary obstruction such as portal hypertensive biliopathy (PHB) that requires not only the relief of the obstructive jaundice but also the diagnosis of the cause of portal hypertension and its management.3

2. Clinical case

A 36-year-old woman presented to the emergency department due to jaundice that had first become apparent three days earlier. She also reported pruritus for the last week, pale stools and dark discoloration of the urine. While denying any abdominal pain, the patient did complain of nausea and vomiting.

At the time of presentation the patient did not recall any significant past medical history including blood transfusions and was taking no prescription or over-the-counter drugs. She also denied unprotected sexual intercourse.

Shellie Toe Botas Pretas Têxteis Curtas Peep Mia Physical examination was notable for marked jaundice and scratch induced excoriations. The gallbladder was not palpable but a nontender hepatomegaly extending 2 cm below the ribcage was noted. The remaining of the physical examination was unremarkable.

The initial laboratory test results consisted of a normal complete blood count and a normal prothrombin time of 10.2 s (control 12.0 s). Total bilirubin was elevated at 9.9 mg/dL (0.3-1.2 mg/dL) with a conjugated bilirubin of 8.7 mg/dL. Alkaline phosphatase was also raised at 237 IU/L (35-105 IU/L) as was aspartate aminotransferase with a value of 170 IU/L (<32 IU/L).

An ultrasound scan of the abdomen was performed and showed dilatation of the intrahepatic biliary ducts as well as a dilated common bile duct measuring 8.5mm in diameter with at least one biliary calculus in its lumen. Gallstones were also present and mild hepatomegaly and splenomegaly were reported. Due to the concern of portal hypertension raised by the presence of splenomegaly, a computed tomography (CT) of the abdomen was ordered and revealed an extensive portosystemic collateral circulation and a portal cavernoma encasing the common bile duct.

At this time the patients mother reported to the attending physician that her daughter had suffered from neonatal sepsis secondary to omphalitis two weeks after birth (Fig. 1).

Endoscopic ultrasonography does not yet have a defined role in PHB and its use is limited to the cases where other imaging tests are unable to give a clear answer, namely in the differential diagnosis of common bile duct stones, calculi or tumors.17

Therapy is currently not recommend for asymptomatic individuals with normal liver function tests. However, patients presenting with symptoms require an integrated management addressing not only biliary obstruction but also portal hypertension. Endoscopic treatment is the therapy of choice when there is choledocholithiasis, acute cholangitis or if shunt surgery is not possible.3,18-21 ERCP is usually effective in stone extraction and treatment of dominant strictures.3,14,18 However, the relief of biliary obstruction with balloon dilation and stenting is only a temporary measure and mandates the construction of a portosystemic venous shunt or regular sessions of endotherapy since stents tend to become clogged over time.14 Increased risk of bleeding is also an issue due to presence of venous collaterals.22

Patients that have a shuntable vein should be offered portosystemic shunt surgery since this procedure effectively addresses portal hypertension and may be the only treatment needed for dominant biliary strictures.23-25Pampa Goma De Wps Ouro Deserto âmbar Manguito Unisexo 228 Meados Botas Ouro Desporto Paládio erwachsene AwBqOF Transjugular intrahepatic portosystemic shunt may be a valid alternative, albeit with less supporting evidence, and may be considered in selected cases when surgery is not feasible or is declined.3 In some cases additional biliary bypass surgery or cholecystectomy may be required.

Since our patient exhibited biliary stones in association with PHB she was initially managed endoscopically with great success and is now awaiting shunt surgery without any symptoms. The need for an effective management of portal hypertension in this patients, even when jaundice has resolved, is emphasized by the diagnosis of secondary biliary cirrhosis and liver dysfunction in patients with longstanding and untreated PHB.16,18

In summary, PHB is an uncommon entity that seldom causes symptoms. However, when symptomatic, the management must be tailored individually and should include shunt surgery. Magnetic resonance is the imaging modality of choice and ERCP is very useful when biliary stones, acute cholangitis or contraindications for surgery are present.

Protection of human and animal subjects. The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data. The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent. The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.